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Kawasaki Disease and Inborn Errors of Immunity: Exploring the Link and Implications
The exact etiopathogenesis of Kawasaki disease (KD), the most common childhood vasculitis, remains unknown; however, an aberrant immune response, possibly triggered by an infectious or environmental agent in genetically predisposed children, is believed to be the underlying pathogenetic mechanism. P...
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10341281/ https://www.ncbi.nlm.nih.gov/pubmed/37443545 http://dx.doi.org/10.3390/diagnostics13132151 |
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author | Sharma, Saniya Nadig, Pallavi L Pilania, Rakesh Kumar Sharma, Kaushal Dhaliwal, Manpreet Rawat, Amit Singh, Surjit |
author_facet | Sharma, Saniya Nadig, Pallavi L Pilania, Rakesh Kumar Sharma, Kaushal Dhaliwal, Manpreet Rawat, Amit Singh, Surjit |
author_sort | Sharma, Saniya |
collection | PubMed |
description | The exact etiopathogenesis of Kawasaki disease (KD), the most common childhood vasculitis, remains unknown; however, an aberrant immune response, possibly triggered by an infectious or environmental agent in genetically predisposed children, is believed to be the underlying pathogenetic mechanism. Patients with inborn errors of immunity (IEI) are predisposed to infections that trigger immune dysregulation due to an imbalance in various arms of the immune system. KD may develop as a complication in both primary and secondary immunodeficiencies. KD may occur either at disease presentation or have a later onset in IEIs. These include X-linked agammaglobulinemia (XLA), selective IgA deficiency, transient hypogammaglobulinemia of infancy; Wiskott–Aldrich syndrome (WAS), hyper IgE syndrome (HIES); chronic granulomatous disease (CGD), innate and intrinsic immunity defects, and autoinflammatory diseases, including PFAPA. Hitherto, the association between KD and IEI is confined to specific case reports and case series and, thus, requires extensive research for a comprehensive understanding of the underlying pathophysiological mechanisms. IEIs may serve as excellent disease models that would open new insights into the disease pathogenesis of children affected with KD. The current review highlights this critical association between KD and IEI supported by published literature. |
format | Online Article Text |
id | pubmed-10341281 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-103412812023-07-14 Kawasaki Disease and Inborn Errors of Immunity: Exploring the Link and Implications Sharma, Saniya Nadig, Pallavi L Pilania, Rakesh Kumar Sharma, Kaushal Dhaliwal, Manpreet Rawat, Amit Singh, Surjit Diagnostics (Basel) Review The exact etiopathogenesis of Kawasaki disease (KD), the most common childhood vasculitis, remains unknown; however, an aberrant immune response, possibly triggered by an infectious or environmental agent in genetically predisposed children, is believed to be the underlying pathogenetic mechanism. Patients with inborn errors of immunity (IEI) are predisposed to infections that trigger immune dysregulation due to an imbalance in various arms of the immune system. KD may develop as a complication in both primary and secondary immunodeficiencies. KD may occur either at disease presentation or have a later onset in IEIs. These include X-linked agammaglobulinemia (XLA), selective IgA deficiency, transient hypogammaglobulinemia of infancy; Wiskott–Aldrich syndrome (WAS), hyper IgE syndrome (HIES); chronic granulomatous disease (CGD), innate and intrinsic immunity defects, and autoinflammatory diseases, including PFAPA. Hitherto, the association between KD and IEI is confined to specific case reports and case series and, thus, requires extensive research for a comprehensive understanding of the underlying pathophysiological mechanisms. IEIs may serve as excellent disease models that would open new insights into the disease pathogenesis of children affected with KD. The current review highlights this critical association between KD and IEI supported by published literature. MDPI 2023-06-23 /pmc/articles/PMC10341281/ /pubmed/37443545 http://dx.doi.org/10.3390/diagnostics13132151 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Review Sharma, Saniya Nadig, Pallavi L Pilania, Rakesh Kumar Sharma, Kaushal Dhaliwal, Manpreet Rawat, Amit Singh, Surjit Kawasaki Disease and Inborn Errors of Immunity: Exploring the Link and Implications |
title | Kawasaki Disease and Inborn Errors of Immunity: Exploring the Link and Implications |
title_full | Kawasaki Disease and Inborn Errors of Immunity: Exploring the Link and Implications |
title_fullStr | Kawasaki Disease and Inborn Errors of Immunity: Exploring the Link and Implications |
title_full_unstemmed | Kawasaki Disease and Inborn Errors of Immunity: Exploring the Link and Implications |
title_short | Kawasaki Disease and Inborn Errors of Immunity: Exploring the Link and Implications |
title_sort | kawasaki disease and inborn errors of immunity: exploring the link and implications |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10341281/ https://www.ncbi.nlm.nih.gov/pubmed/37443545 http://dx.doi.org/10.3390/diagnostics13132151 |
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